Clear cell carcinoma of the cervix and vagina is rare in the pediatric population. Many of these tumors are associated with prior intrauterine diethylstilbestrol (DES) exposure. All DES-associated tumors are believed to contain microsatellite instability (MI). Historically, the recommended treatment is radical hysterectomy and pelvic lymphadenectomy, which results in infertility in all cases. Radical abdominal or vaginal trachelectomy and pelvic lymph node dissection is a new technique utilized in adult women with early cervical cancer who wish to retain fertility. This novel approach is also pertinent to the pediatric patient and is described in this report. A molecular analysis is also performed to determine if these are DES-associated tumors.

Due to the narrow vaginal anatomy in pediatric patients a vaginal approach is not possible, and an abdominal approach is performed. The resection includes the cervix, upper vagina, parametrium, and paracolpos. Pelvic lymphadenectomy is performed in a similar manner to the adult patient. Matched pairs of normal and tumor DNA from both cases were examined for evidence of MI using a consensus panel of microsatellite markers.

Two girls aged 6 and 8 years and without history of DES exposure presented with vaginal bleeding. Vaginoscopy revealed cervical polyp in both cases. Biopsies demonstrated clear cell cancer stage IB1 in both patients. They underwent radical abdominal trachelectomy and bilateral pelvic lymph node dissection along with anastomosis of uterine isthmus to upper vagina. Intraoperative frozen-section analysis confirmed negative uterine and vaginal margins. No adjuvant treatment was given and both girls remain disease free. Neither tumor showed evidence for MI, confirming that these are not DES-associated tumors.

To our knowledge, this is the first report of radical abdominal trachelectomy in the pediatric age group and it is likely to include the youngest patient with clear cell carcinoma of the uterine cervix not associated with DES exposure. This novel approach is feasible and appears safe in the pediatric age group.